Clinical experience of seropositive ganglionic acetylcholine receptor antibody in a tertiary neurology referral center

Abstract

Detection of gAChR-Ab, especially at a higher level, is helpful for the diagnosis of AAG in patients with corresponding autonomic symptoms. However, its value is limited for predicting cancer risk and for diagnosis and management of patients without autonomic symptoms.

Ganglionic Antibody Level as a Predictor of Severity of Autonomic Failure

“…Our study and that of Li et al (2015) 30 also shows that high ganglionic AChR Ab level is not required for severe AF (i.e., low level can be associated with more than mild or moderate autonomic impairment). This distinction from earlier reports is likely due to the different methods of case ascertainment.…”

“…13 Our study confirms prior reports associating higher level with more severe AF. 3,5,8,13,25,29,30 This correlation was observed in all three subdomains of the CASS score – adrenergic, cardiova gal, and sudomotor – and reflects the presence of the ganglionic AChR Ab in all types of autonomic ganglia. 31…”

Is postural tachycardia syndrome an autoimmune disorder? And other updates on recent autonomic research

“…However, some patients with POTS also tested positive with low titers [13]. Autonomic experts now agree that positivity for ganglionic nicotinic antibodies at low titer (i.e., below 0.2 nmol/L) has no clinical relevance for POTS, as these can be detected in up to 5% of healthy controls [9,12,14].…”

Autoantibody diversity in paraneoplastic syndromes and related disorders: The need for a more guided screening approach

“…In addition to anti-VGKC for acquired neuromyotonia (Isaac's syndrome), other major autoantibodies include voltage gated calcium channel (VGCC) associated with Lambert-Eaton myasthenic syndrome, striated muscle (STM) and acetylcholine receptor (AChR) binding and blocking (myasthenia gravis, MG), ganglionic nicotinic acetylcholine receptor (gAChR) in patients with autoimmune autonomic ganglionopathy [11][12][13]. Recently, antibodies directed against dipeptidyl peptidase-like protein 6 (DPPX), a regulatory subunit of the Kv4.2 potassium channels on the surface of neurons were reported in a novel and distinct variant of progressive encephalomyelitis with rigidity and myoclonus (PERM) [14].…”